Sinonazal rabdomiyosarkom

Özet

Sinonazal rabdomiyosarkom, özellikle çocuklarda nazal kavite ve paranazal sinüslerden kaynaklanabilen agresif yumuşak doku sarkomudur. Kontrastsız BT kemik destrüksiyonu ve sinüs duvarı etkisini, kontrastlı inceleme kitlenin sınırlarını ve orbita ya da kafa tabanı komşuluğunu ortaya koyar. BT ve MR yumuşak doku ile intrakraniyal uzanımı haritalamak için kullanılır; bazı görüntü bulguları alt tiplerle ilişkili olabilir (embriyonelde botrioid boyanma, alveolerde kafa tabanı tutulumu) ancak kesin alt tip tayini histopatolojiye dayanır. Orbita, kafa tabanı veya nazofarenks uzanımının atlanması lokal hastalık yaygınlığını olduğundan az gösterir.

Faz ve pencere

Kontrastsız tanısal

Kemik algoritmasında nazal kavite ve etmoid çevresindeki lamina papyracea, etmoid çatı ve sfenoid kemikte permeatif ya da düzensiz destrüksiyon haritalanır; kitle merkezi ve sinüs dolumu da seçilir.

Kontrastlı tanısal

Post-IV kontrastlı yumuşak doku serisinde solid kitlenin değişken, bazen heterojen kontrastlanması ile orbital yağ, pterigopalatin fossa, nazofarenks ve kafa tabanı komşuluğuna uzanım belirlenir; histolojik alt tip kontrastlanma ile tayin edilemez.

Önerilen pencereler: Yumuşak doku (G 400 / M 40), Kemik (G 1800 / M 400).

BT bulguları

• Nazal kavite, etmoid hücreler veya nazofarenkste agresif yumuşak doku kitlesi.
• Konkalar, nazal septum veya etmoid lamellerde destrüktif/permeatif kemik kaybı.
• Lamina papyracea geçişiyle medial orbita yumuşak dokusuna uzanım.
• Sfenoid kemik, kribriform plak ya da kafa tabanında erozyon ve komşu kafa içi yayılım kuşkusu.
• Kontrastlı görüntüde heterojen veya belirgin boyanan solid kitle.
• Kitle çevresinde doku planlarının silinmesi ve nazofarenks/derin yüz boşluklarına uzanım.

Normalde

Normal etmoid hücreler havalıdır; lamina papyracea, nazal septum ve kafa tabanı konturları kesintisiz ince kemik çizgileri oluşturur. Karşı taraf etmoid ve orbita duvarıyla kıyasla kemik kaybının yönünü belirle, ardından medial orbital yağ ile kafa içi yumuşak dokunun korunup korunmadığını ara.

Ayırıcı tanı

Sinonazal lenfoma

yumuşak doku infiltrasyonu benzer olabilir, fakat kemik değişikliği ve dağılım farklılık gösterebilir.

Ewing sarkomu

çocukta kemik merkezli destrüktif kitle; sinonazal yerleşimde görüntü örtüşebilir.

Sinonazal nöroblastom

olfaktör oluk ve kribriform plak merkezli üst nazal kitle.

Skuamöz hücreli karsinom

daha çok erişkinde, sinüs duvarında belirgin destrüksiyonla görülür.

İnvaziv fungal sinüzit

immün baskılanma, nekroz ve sinüs dışı yağ planı infiltrasyonu tanıyı destekler.

Tuzaklar

• Kitle içi nekroz veya tıkanmış sinüs sekresyonu heterojenliği artırabilir; kontrastlanan solid kenarı ayrıca izle.
• Kemik yıkımının olmaması erken tümörü dışlamaz; yumuşak doku kitlesi ve kritik komşulukları değerlendirmek gerekir.

Kendini dene

1. Çocukta etmoid merkezli agresif kitle lamina papyracea'yı yıkıp orbitaya uzanıyor. En olası malignite hangisidir?

   • Rabdomiyosarkom
   • Osteom
   • Antrokoanal polip
   • Konka bülloza
   Cevabı göster

   Rabdomiyosarkom. Çocukta agresif sinonazal yumuşak doku kitlesi ve destrüktif komşuluk rabdomiyosarkom olasılığını yükseltir; kemik kökenli osteom bu davranışı göstermez.

2. Üst nazal kaviteden kribriform plak üzerinden kafa içine uzanan kitle olfaktör olukta merkezli. En yakın ayırıcı?

   • Olfaktör nöroblastom (estesiyonöroblastom)
   • Antrolit
   • Konka hipertrofisi
   • Maksiller mukosel
   Cevabı göster

   Olfaktör nöroblastom (estesiyonöroblastom). Olfaktör oluk ve kribriform plak merkezli yayılım olfaktör nöroblastomun (estesiyonöroblastomun) tipik başlangıç güzergâhıdır.

İlgili konular

• Estesiyonöroblastom (olfaktör nöroblastom)

Kaynaklar

Bu sayfadaki 32 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 1 cümle bu karşılaştırmada düzeltildi (2026-10-08).

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